Review
Clinical assessment of Tourette syndrome and tic disorders

https://doi.org/10.1016/j.neubiorev.2012.11.013Get rights and content

Abstract

Tourette syndrome (TS) is a neuropsychiatric disorder involving multiple motor and phonic tics. Tics, which usually begin between the ages of 6 and 8, are sudden, rapid, stereotyped, and apparently purposeless movements or sounds that involve discrete muscle groups. Individuals with TS experience a variety of different sensory phenomena, including premonitory urges prior to tics and somatic hypersensitivity due to impaired sensorimotor gating. In addition to other conditions, stress, anxiety, fatigue, or other heightened emotional states tend to exacerbate tics, while relaxation, playing sports, and focused concentration on a specific task tend to alleviate tic symptoms. Ninety percent of children with TS also have comorbid conditions, such as attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), or an impulse control disorder. These disorders often cause more problems for the child both at home and at school than tics do alone. Proper diagnosis and treatment of TS involves appropriate evaluation and recognition, not only of tics, but also of these associated conditions.

Highlights

► Proper Tourette syndrome assessment must include evaluation of common comorbidities. ► Most patients with Tourette syndrome experience premonitory urges prior to tics. ► Rating scales exist to measure severity of tics and associated sensory phenomena. ► Most children with Tourette syndrome improve during adolescence and early adulthood.

Introduction

Tourette syndrome (TS) was first described by the French neurologist, Gilles de la Tourette, in 1885 as a “maladie des tics.” In his original case series describing the syndrome that now bears his name, Gilles de la Tourette wrote about many of the characteristics of the syndrome including: involuntary movements and sounds, markedly enhanced startle reactions, a tendency to repeat both vocalizations (echolalia) and movements (echopraxia), and uncontrollable verbal obscenities (coprolalia) (Lajonchere et al., 1996). Since then, our knowledge of TS has progressed significantly, including advances in our understanding of tics, their surrounding sensory phenomena, and the central role that other co-occurring diseases, such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), have on the overall clinical course of the disorder. This review will focus on our current understanding of the diagnosis, clinical characterization and assessment of tics as well as their clinical course. Other reviews will focus on the evidence-based treatment and neurobiology of tic disorders.

Section snippets

Definition of tics

Tics appear as sudden, rapid, purposeless motor movements or sounds that involve discrete muscle groups. They are also stereotyped in that they will occur in a similar manner each time they are performed. In comparison to some movement disorders or psychiatric conditions (e.g. sterotypies, chorea, or dyskinesia), patients with tics report the ability to suppress them, even if only for a short duration. However, they report that suppression often causes discomfort. Almost any movement, sound, or

Tourette syndrome and other tic disorders

The prevalence of TS varies based on study design and location. An international prevalence of 0.6–1% has been reported for mainstream schoolchildren, with the disorder being 3–4 times more common in males than in females (Cavanna and Termine, 2012). Data from the 2007 National Survey of Children's Health (NSCH) showed an estimated prevalence of 0.3% among U.S. children aged 6–17 years (Scahill et al., 2009). This number may represent an underestimate of TS prevalence since data were gathered

Characterization of tics

Tics are characterized by their anatomical location, number, frequency, and duration. They are also further described by their forcefulness or intensity and by their complexity (ranging from simple to complex). The most widely-used rating scale of tic severity is the Yale Global Tic Severity Scale (YGTSS), which includes separate scores from 0 to 5 for number, frequency, intensity, complexity, and interference (the degree to which planned actions or speech are interrupted by tics) of both motor

Natural history

The natural history of TS has been established based on clinical observations. There is a clear progression of the disorder from the onset of symptoms to, in most cases, full or partial regression of symptoms. Tics usually begin around 6–8 years of age, and 90–95% of TS cases have an onset of tics between the ages of 4 and 13 (Leckman et al., 1998). Simple motor tics involving the eyes or face are usually the first to appear in a child with TS. They are called simple because they involve a

Sensory phenomena surrounding tics

The outward manifestation of TS represents only a part of the symptomatology experienced by most of our patients. In 1980, Joseph Bliss, articulately described his careful observations from 35 years of self-study of the feelings and subjective events surrounding his own tics. Much of what he described became the basis for future research surrounding the sensory phenomena associated with tics. The term, “sensory phenomena,” is now used as an all-encompassing term to describe such subjective

Exacerbating/alleviating factors

Tic symptoms vary in frequency and intensity, and in addition to potential neurological variation, it has been shown that certain environmental or contextual factors will either exacerbate or alleviate tic symptoms in individuals with TS.

The results of 6 different descriptive studies looking at the effects of different antecedent variables on tic severity show stress and anxiety appear to be the most common factors associated with an increase in TS symptoms, while fatigue and boredom also rank

Suppressing tics

One of the characteristics of tic symptoms is that they are suppressible, even if only for a short while. However, as stated earlier, the act of suppression can lead to the build-up of uncomfortable premonitory urges. In one study, 3 of 4 children who demonstrated reliable suppression showed a pattern of higher subjective urge ratings during suppression as compared to baseline (Himle et al., 2007).

Although tics can be suppressed, to do so requires more attention and energy from the individual.

Comorbidities

The description of behavioral and emotional disturbances in patients with TS has occurred since 1899, around the time the disorder was first described by Georges Gilles de la Tourette himself (Coffey and Park, 1997). In fact, comorbid neuropsychiatric disorders, the majority being ADHD and OCD, have been shown to occur in up to 90% of TS patients in both clinic and community settings (Wright et al., 2012). Figure 2 depicts the time course of common comorbidities in relation to tic symptoms, as

Conclusions

Tourette's syndrome is a neuropsychiatric disorder characterized by multiple motor and vocal tics. However, for many individuals with TS, the tics are neither the most prominent nor distressing part of the disorder. In the majority of children with TS, tic symptoms diminish significantly during adolescence. Most individuals with TS experience associated sensory phenomena such as premonitory urges and somatic hypersensitivity that are often as distressing as the tics themselves. The majority of

Conflict of interest

The authors have no conflicts of interest to disclose.

Acknowledgements

The authors acknowledge the National Institute of Mental Health support of the Trichotillomania Learning Center (MHB), the Yale Child Study Center Research Training Program (MHB), the National Institutes of Health (K23MH091240 (MHB), T32MH018268-26 (JFL) and R25 MH077823 (JFL)), the APIRE/Eli Lilly Psychiatric Research Fellowship (MHB), the AACAP/Eli Lilly Junior Investigator Award (MHB), NARSAD (MHB), and UL1 RR024139 from the National Center for Research Resources, a component of the National

References (91)

  • J. Jankovic

    Tourette syndrome. Phenomenology and classification of tics

    Neurologic Clinics

    (1997)
  • M.J. Kane

    Premonitory urges as “attentional tics” in Tourette's syndrome

    Journal of the American Academy of Child and Adolescent Psychiatry

    (1994)
  • J.F. Leckman

    Tourette's syndrome

    Lancet

    (2002)
  • J.F. Leckman et al.

    The Yale Global Tic Severity Scale: initial testing of a clinician-rated scale of tic severity

    Journal of the American Academy of Child and Adolescent Psychiatry

    (1989)
  • A.B. Lewin et al.

    Comparison of clinical features among youth with tic disorders, obsessive-compulsive disorder (OCD), and both conditions

    Psychiatry Research

    (2010)
  • J.S. March et al.

    Tics moderate treatment outcome with sertraline but not cognitive-behavior therapy in pediatric obsessive-compulsive disorder

    Biological Psychiatry

    (2007)
  • Y. Nagai et al.

    Influence of sympathetic autonomic arousal on tics: implications for a therapeutic behavioral intervention for Tourette syndrome

    Journal of Psychosomatic Research

    (2009)
  • S. Pliszka

    Practice parameter for the assessment and treatment of children and adolescents with attention-deficit/hyperactivity disorder

    Journal of the American Academy of Child and Adolescent Psychiatry

    (2007)
  • M.M. Robertson

    The prevalence and epidemiology of Gilles de la Tourette syndrome. Part 1: the epidemiological and prevalence studies

    Journal of Psychosomatic Research

    (2008)
  • M.M. Robertson

    The prevalence and epidemiology of Gilles de la Tourette syndrome. Part 2: tentative explanations for differing prevalence figures in GTS, including the possible effects of psychopathology, aetiology, cultural differences, and differing phenotypes

    Journal of Psychosomatic Research

    (2008)
  • A.K. Shapiro et al.

    Controlled study of pimozide vs. placebo in Tourette's syndrome

    Journal of the American Academy of Child Psychiatry

    (1984)
  • H.S. Singer et al.

    Development of behavioral and emotional problems in Tourette syndrome

    Pediatric Neurology

    (1989)
  • D.G. Sukhodolsky et al.

    Disruptive behavior in children with Tourette's syndrome: association with ADHD comorbidity, tic severity, and functional impairment

    Journal of the American Academy of Child and Adolescent Psychiatry

    (2003)
  • J.T. Walkup et al.

    The validity of instruments measuring tic severity in Tourette's syndrome

    Journal of the American Academy of Child and Adolescent Psychiatry

    (1992)
  • T. Banaschewski et al.

    Premonitory sensory phenomena and suppressibility of tics in Tourette syndrome: developmental aspects in children and adolescents

    Developmental Medicine & Child Neurology

    (2003)
  • H.N. Bawden et al.

    Peer relationship problems in children with Tourette's disorder or diabetes mellitus

    Journal of Child Psychology and Psychiatry and Allied Disciplines

    (1998)
  • B.A. Belluscio et al.

    Sensory sensitivity to external stimuli in Tourette syndrome patients

    Movement Disorders

    (2011)
  • B.A. Belluscio et al.

    Similarities and differences between normal urges and the urge to tic

    Cognitive Neuroscience

    (2011)
  • J. Bliss

    Sensory experiences of Gilles de la Tourette syndrome

    Archives of General Psychiatry

    (1980)
  • M.H. Bloch et al.

    A systematic review: antipsychotic augmentation with treatment refractory obsessive-compulsive disorder

    Molecular Psychiatry

    (2006)
  • M.H. Bloch et al.

    Caudate volumes in childhood predict symptom severity in adults with Tourette syndrome

    Neurology

    (2005)
  • M.H. Bloch et al.

    Adulthood outcome of tic and obsessive-compulsive symptom severity in children with Tourette syndrome

    Archives of Pediatrics and Adolescent Medicine

    (2006)
  • M.H. Bloch et al.

    Fine-motor skill deficits in childhood predict adulthood tic severity and global psychosocial functioning in Tourette's syndrome

    Journal of Child Psychology and Psychiatry and Allied Disciplines

    (2006)
  • C.L. Budman et al.

    Rage attacks in children and adolescents with Tourette's disorder: a pilot study

    Journal of Clinical Psychiatry

    (1998)
  • W.E. Bunney et al.

    Structured Interview for Assessing Perceptual Anomalies (SIAPA)

    Schizophrenia Bulletin

    (1999)
  • A.S. Carter et al.

    Social and emotional adjustment in children affected with Gilles de la Tourette's syndrome: associations with ADHD and family functioning. Attention deficit hyperactivity disorder

    Journal of Child Psychology and Psychiatry and Allied Disciplines

    (2000)
  • A.M. Catafau et al.

    Relationship between cerebral perfusion in frontal-limbic-basal ganglia circuits and neuropsychologic impairment in patients with subclinical hepatic encephalopathy

    Journal of Nuclear Medicine

    (2000)
  • A.E. Cavanna et al.

    Tourette syndrome

    Advances in Experimental Medicine & Biology

    (2012)
  • A.J. Cohen et al.

    Sensory phenomena associated with Gilles de la Tourette's syndrome

    Journal of Clinical Psychiatry

    (1992)
  • DSM-IV-TR

    Diagnostic and statistical manual of mental disorders: DSM-IV-TR

    (2000)
  • C.M. Eddy et al.

    Clinical correlates of quality of life in Tourette syndrome

    Movement Disorders

    (2011)
  • R.D. Freeman

    Tic disorders and ADHD: answers from a world-wide clinical dataset on Tourette syndrome

    European Child and Adolescent Psychiatry

    (2007)
  • R.D. Freeman et al.

    Coprophenomena in Tourette syndrome

    Developmental Medicine and Child Neurology

    (2009)
  • W.P. Hetrick et al.

    Phenomenological dimensions of sensory gating

    Schizophrenia Bulletin

    (2012)
  • M.B. Himle et al.

    Establishing the feasibility of direct observation in the assessment of tics in children with chronic tic disorders

    Journal of Applied Behavior Analysis

    (2006)
  • Cited by (154)

    • Technologies for the management of hyperkinetic movement disorders

      2024, Handbook of Digital Technologies in Movement Disorders
    View all citing articles on Scopus
    View full text